Found 1937 results
Signes A, Cerutti R, Dickson AS, Benincá C, Hinchy EC, Ghezzi D, Carrozzo R, Bertini E, Murphy MP, Nathan JA, Viscomi C, Fernandez-Vizarra E & Zeviani M (2019) APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS. EMBO Mol Med 11
Santibanez-Koref M, Griffin H, Turnbull DM, Chinnery PF, Herbert M & Hudson G (2019) Assessing mitochondrial heteroplasmy using next generation sequencing: A note of caution. Mitochondrion 46, 302-306
Viscomi C & Zeviani M (2019) Breathe: Your Mitochondria Will Do the Rest… If They Are Healthy!. Cell Metabolism 30, 628 - 629
Tufi R, Gleeson TP, von Stockum S, Hewitt VL, Lee JJ, Terriente-Felix A, Sanchez-Martinez A, Ziviani E & Whitworth AJ (2019) Comprehensive Genetic Characterization of Mitochondrial Ca Uniporter Components Reveals Their Different Physiological Requirements In Vivo. Cell Rep 27, 1541-1550.e5
Boczonadi V, King MS, Smith AC, Olahova M, Bansagi B, Roos A, Eyassu F, Borchers C, Ramesh V, Lochmüller H, Polvikoski T, Whittaker RG, Pyle A, Griffin H, Taylor RW, Chinnery PF, Robinson AJ, Kunji ERS & Horvath R (2019) Correction: Mitochondrial oxodicarboxylate carrier deficiency is associated with mitochondrial DNA depletion and spinal muscular atrophy-like disease. Genet Med
Kohlhauer M, Pell VR, Burger N, Spiroski AM, Gruszczyk A, Mulvey JF, Mottahedin A, Costa ASH, Frezza C, Ghaleh B, Murphy MP, Tissier R & Krieg T (2019) Correction to: Protection against cardiac ischemia-reperfusion injury by hypothermia and by inhibition of succinate accumulation and oxidation is additive. Basic Res Cardiol 114, 24
Ryan DG, Murphy MP, Frezza C, Prag HA, Chouchani ET, O'Neill LA & Mills EL (2019) Coupling Krebs cycle metabolites to signalling in immunity and cancer. Nat Metab 1, 16-33
Sobti M, Ishmukhametov R, Bouwer JC, Ayer A, Suarna C, Smith NJ, Christie M, Stocker R, Duncan TM & Stewart AG (2019) Cryo-EM reveals distinct conformations of ATP synthase on exposure to ATP. Elife 8
Gu J, Zhang L, Zong S, Guo R, Liu T, Yi J, Wang P, Zhuo W & Yang M (2019) Cryo-EM structure of the mammalian ATP synthase tetramer bound with inhibitory protein IF1. Science 364, 1068-1075
Chinnery PF & Prudent J (2019) De-fusing mitochondria defuses the mtDNA time-bomb. Cell Res
Domínguez-González C, Madruga-Garrido M, Mavillard F, Garone C, Aguirre-Rodríguez FJavier, M Donati A, Kleinsteuber K, Martí I, Martín-Hernández E, Morealejo-Aycinena JP, Munell F, Nascimento A, Kalko SG, M Sardina D, Del Vayo CÁlvarez, Serrano O, Long Y, Tu Y, Levin B, Thompson JLP, Engelstad K, Uddin J, Torres-Torronteras J, Jimenez-Mallebrera C, Martí R, Paradas C & Hirano M (2019) Deoxynucleoside Therapy for Thymidine Kinase 2-Deficient Myopathy. Ann Neurol
Lau GY, Barts N, Hartley RC, Tobler M, Richards JG, Murphy MP & Arndt S (2019) Detection of changes in mitochondrial hydrogen sulfide in the fish model (Poeciliidae). Biol Open 8
Parikh S, Karaa A, Goldstein A, Bertini ESilvio, Chinnery PF, Christodoulou J, Cohen BH, Davis RL, Falk MJ, Fratter C, Horvath R, Koenig MKay, Mancuso M, McCormack S, McCormick EM, McFarland R, Nesbitt V, Schiff M, Steele H, Stockler S, Sue C, Tarnopolsky M, Thorburn DR, Vockley J & Rahman S (2019) Diagnosis of 'possible' mitochondrial disease: an existential crisis. J Med Genet 56, 123-130
Ruprecht J & Kunji E (2019) Editorial overview: COSB Membranes. Curr Opin Struct Biol 57, vi-viii
Hoitzing H, Gammage PA, Van Haute L, Minczuk M, Johnston IG & Jones NS (2019) Energetic costs of cellular and therapeutic control of stochastic mitochondrial DNA populations. PLoS Comput Biol 15, e1007023
Nieminuszczy J, Broderick R, Bellani MA, Smethurst E, Schwab RA, Cherdyntseva V, Evmorfopoulou T, Lin Y-L, Minczuk M, Pasero P, Gagos S, Seidman MM & Niedzwiedz W (2019) EXD2 Protects Stressed Replication Forks and Is Required for Cell Viability in the Absence of BRCA1/2. Mol Cell
Boreikaite V, Wicky BIM, Watt IN, Clarke J & Walker JE (2019) Extrinsic conditions influence the self-association and structure of IF, the regulatory protein of mitochondrial ATP synthase. Proc Natl Acad Sci U S A 116, 10354-10359
Guyatt AL, Brennan RR, Burrows K, Guthrie PAI, Ascione R, Ring SM, Gaunt TR, Pyle A, Cordell HJ, Lawlor DA, Chinnery PF, Hudson G & Rodriguez S (2019) A genome-wide association study of mitochondrial DNA copy number in two population-based cohorts. Hum Genomics 13, 6
Wei W, Tuna S, Keogh MJ, Smith KR, Aitman TJ, Beales PL, Bennett DL, Gale DP, Bitner-Glindzicz MAK, Black GC, Brennan P, Elliott P, Flinter FA, R Floto A, Houlden H, Irving M, Koziell A, Maher ER, Markus HS, Morrell NW, Newman WG, Roberts I, Sayer JA, Smith KGC, Taylor JC, Watkins H, Webster AR, Wilkie AOM, Williamson C, Ashford S, Penkett CJ, Stirrups KE, Rendon A, Ouwehand WH, Bradley JR, F Raymond L, Caulfield M, Turro E & Chinnery PF (2019) Germline selection shapes human mitochondrial DNA diversity. Science 364
Sommerville EW, Rosa IDalla, Rosenberg MM, Bruni F, Thompson K, Rocha M, Blakely EL, He L, Falkous G, Schaefer AM, Yu-Wai-Man P, Chinnery PF, Hedstrom L, Spinazzola A, Taylor RW & Gorman GS (2019) Identification of a novel heterozygous guanosine monophosphate reductase (GMPR) variant in a patient with a late-onset disorder of mitochondrial DNA maintenance. Clin Genet
Mohanraj K, Wasilewski M, Benincá C, Cysewski D, Poznanski J, Sakowska P, Bugajska Z, Deckers M, Dennerlein S, Fernandez-Vizarra E, Rehling P, Dadlez M, Zeviani M & Chacinska A (2019) Inhibition of proteasome rescues a pathogenic variant of respiratory chain assembly factor COA7. EMBO Mol Med 11
von Stockum S, Sanchez-Martinez A, Corrà S, Chakraborty J, Marchesan E, Locatello L, Da Re C, Cusumano P, Caicci F, Ferrari V, Costa R, Bubacco L, Rasotto MBerica, Szabó I, Whitworth AJ, Scorrano L & Ziviani E (2019) Inhibition of the deubiquitinase USP8 corrects a Drosophila PINK1 model of mitochondria dysfunction. Life Sci Alliance 2
Brischigliaro M, Corrà S, Tregnago C, Fernandez-Vizarra E, Zeviani M, Costa R & De Pittà C (2019) Knockdown of APOPT1/COA8 Causes Cytochrome Oxidase Deficiency, Neuromuscular Impairment, and Reduced Resistance to Oxidative Stress in Drosophila melanogaster. Front Physiol 10, 1143
Małecki JM, Willemen HLDM, Pinto R, Y Y Ho A, Moen A, Kjønstad IF, Burgering BMT, Zwartkruis F, Eijkelkamp N & Falnes PØ (2019) Lysine methylation by the mitochondrial methyltransferase FAM173B optimizes the function of mitochondrial ATP synthase. J Biol Chem 294, 1128-1141
Galván-Peña S, Carroll RG, Newman C, Hinchy EC, Palsson-McDermott E, Robinson EK, Covarrubias S, Nadin A, James AM, Haneklaus M, Carpenter S, Kelly VP, Murphy MP, Modis LK & O'Neill LA (2019) Malonylation of GAPDH is an inflammatory signal in macrophages. Nat Commun 10, 338